{"orpha":36236,"name":"Staphylococcal scalded skin syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Generalized exfoliative disease","SSSS"],"definition":"A rare staphylococcal toxemia caused by epidermolytic toxins of <i>Staphylococcus aureus</i> and characterized by the appearance of widespread erythematous patches, on which large blisters develop. Upon rupture of these blisters, the skin appears reddish and scalded. The lesions typically begin in the face and rapidly expand to other parts of the body. The disease may be complicated by pneumonia and sepsis. It most commonly affects newborns and infants.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-36236/","orphanet_url":"https://www.orpha.net/en/disease/detail/36236","names":{"fr":{"name":"Syndrome d'épidermolyse staphylococcique","synonyms":["Syndrome de la peau ébouillantée","Maladie exfoliante généralisée"],"definition":"Infection toxinique staphylococcique rare causée par les toxines épidermolytiques de <i>Staphylococcus aureus</i> et caractérisée par l'apparition de plaques érythémateuses étendues, sur lesquelles se développent de grandes cloques. Lorsqu'elles se rompent, les cloques font apparaître une peau rougeâtre et échaudée. De manière générale, les lésions apparaissent d'abord sur le visage pour s'étendre rapidement à d'autres parties du corps. Une pneumonie et une septicémie font partie des complications. La maladie affecte le plus souvent les nouveau-nés et les nourrissons."},"de":{"name":"Staphylococcal-Scalded-Skin-Syndrom","synonyms":["Dermatitis exfoliativa neonatorum Ritter von Rittershain","SSS-Syndrom","SSSS","Staphylogene toxische epidermale Nekrolyse","Staphylogenes Lyell-Syndrom","Syndrom der verbrühten Haut"],"definition":"Eine seltene Staphylokokken-Toxämie, die durch epidermolytische Toxine von <i>Staphylococcus aureus</i> verursacht wird und durch das Auftreten ausgedehnter erythematöser Flecken gekennzeichnet ist, auf denen sich große Blasen entwickeln. Wenn diese Blasen aufplatzen, erscheint die Haut rötlich und verbrüht. Die Läsionen beginnen typischerweise im Gesicht und breiten sich rasch auf andere Körperteile aus. Die Krankheit kann durch Lungenentzündung und Sepsis kompliziert werden. Am häufigsten sind Neugeborene und Säuglinge betroffen."},"es":{"name":"Síndrome de la piel escaldada por estafilococos","synonyms":["Enfermedad exfoliativa generalizada","SSSS"],"definition":"Es una toxemia estafilocócica poco frecuente causada por las toxinas epidermolíticas de <i>Staphylococcus aureus</i> y caracterizada por la aparición de placas eritematosas generalizadas, en las que se desarrollan grandes ampollas. Al romperse estas ampollas, la piel se torna rojiza y escamada. Por lo general, las lesiones comienzan en la cara, expandiéndose rápidamente a otras partes del cuerpo. La enfermedad puede complicarse por neumonía y sepsis. Afecta con mayor frecuencia a neonatos y lactantes."},"it":{"name":"Sindrome cutanea combustiforme da stafilococco","synonyms":["Malattia esfoliativa generalizzata","SSSS"],"definition":"Si tratta di una tossiemia stafilococcica rara, causata dalle tossine epidermolitiche dello <i>Staphylococcus aureus</i>, caratterizzata da macchie eritematose diffuse, sulle quali si sviluppano ampie vesciche. Alla rottura delle vesciche, la pelle appare rossastra e scottata. Di solito le lesioni interessano dapprima il viso e si diffondono rapidamente ad altre parti del corpo. Le complicanze comprendono la polmonite e la sepsi. La malattia colpisce più comunemente i neonati e i bambini piccoli."},"nl":{"name":"Staphylococcal scalded skin-syndroom","synonyms":["Gegeneraliseerde exfoliatieve ziekte","SSSS"],"definition":"Een zeldzame toxemie door stafylokokken, veroorzaakt door epidermolytische toxines van <i>Staphylococcus aureus</i>, en gekarakteriseerd door verschijnen van wijdverspreide erythemateuze plaques waarop grote blaren ontwikkelen. Bij ruptuur van deze blaren lijkt de huid roodachtig en verschroeid. De laesies verschijnen doorgaans eerst in het aangezicht en breiden dan snel uit naar andere delen van het lichaam. De ziekte kan gecompliceerd worden door pneumonie en sepsis. Vooral neonaten en zuigelingen worden getroffen."},"pt":{"name":"Doença exfoliativa generalizada"},"pl":{"name":"Gronkowcowy zespół oparzeniowy skóry","synonyms":["SSSS","Uogólniona choroba złuszczająca"]},"cs":{"name":"Stafylokokový syndrom opařené kůže","synonyms":["Generalizovaná exfoliativní dermatitida","SSSS"]},"uk":{"name":"Стафілококовий синдром обпеченої шкіри","synonyms":["Генералізована ексфоліативна хвороба","SSSS"],"definition":"A rare staphylococcal toxemia caused by epidermolytic toxins of <i>Staphylococcus aureus</i> and characterized by the appearance of widespread erythematous patches, on which large blisters develop. Upon rupture of these blisters, the skin appears reddish and scalded. The lesions typically begin in the face and rapidly expand to other parts of the body. The disease may be complicated by pneumonia and sepsis. It most commonly affects newborns and infants."},"zh":{"name":"葡萄球菌性烫伤样皮肤综合征","synonyms":["全身性剥脱性疾病"]},"tr":{"name":"Stafilokokal haşlanmış deri sendromu","synonyms":["SSSS","Jeneralize eksfolyatif hastalık"]}},"xrefs":[{"source":"ICD-10","code":"L00","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"EA50.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1554593739"},{"source":"MONDO","code":"0018181","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D013206","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0038165","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10041929","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":300579,"name":"Staphylococcal toxemia","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.01,"area":"Germany","status":"Not yet validated","source":"15816826[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}